Yamaguchi Tadanori, Imamura Yoshiaki, Yamamoto Tatsuya, Fukuda Masaru
Department of Cytopathology, Maizuru Kyosai Hospital, Kyoto, Department of Surgical Pathology, Fukui Medical University Hospital, Fukui, Japan.
Pathol Int. 2003 Mar;53(3):179-85. doi: 10.1046/j.1440-1827.2003.01452.x.
Leiomyomatosis peritonealis disseminata (LPD) is a rare clinicopathological entity typically observed in women of reproductive age. We report a case of LPD with malignant change in a man. A 77-year-old man presented with a mass measuring 10 cm in diameter at the terminal ileum and numerous peritoneal small nodules that were revealed by abdominal computed tomography. Right hemicolectomy with lymph node dissection was performed. Macroscopically, a tumor of the terminal ileum consisted of aggregates of small nodular lesions with calcification and necrosis. The wall of the ileum and colon was intact. Microscopically, some of the nodular lesions consisted of neoplastic growths of atypical spindle cells with cellular atypism and abnormal mitoses. A few of these lesions were completely surrounded by smooth muscle bundles. Hemorrhages and necroses were found within the tumor nodules. Immunohistochemically, the tumor cells were positive for vimentin, desmin, muscle actin, alpha-smooth muscle actin, cytokeratin and p53. The remaining nodular lesions, including small peritoneal lesions, were composed of hypocellular hyalinizing nodules. This case was thought to be LPD with malignant change, although the pathogenesis was uncertain because the tumor cells were negative for estrogen and progesterone receptors.
播散性腹膜平滑肌瘤病（LPD）是一种罕见的临床病理实体，通常见于育龄女性。我们报告一例男性LPD伴恶性变的病例。一名77岁男性因腹部计算机断层扫描发现回肠末端有一个直径10厘米的肿块以及许多腹膜小结节而就诊。行右半结肠切除术并清扫淋巴结。大体上，回肠末端肿瘤由伴有钙化和坏死的小结节状病变聚集体组成。回肠和结肠壁完整。显微镜下，部分结节状病变由具有细胞异型性和异常核分裂的非典型梭形细胞肿瘤性生长构成。其中一些病变完全被平滑肌束包围。肿瘤结节内可见出血和坏死。免疫组化显示，肿瘤细胞波形蛋白、结蛋白、肌动蛋白、α平滑肌肌动蛋白、细胞角蛋白和p53均呈阳性。其余结节状病变，包括小的腹膜病变，由细胞稀少的透明变性结节组成。尽管由于肿瘤细胞雌激素和孕激素受体均为阴性，其发病机制尚不确定，但该病例被认为是LPD伴恶性变。
